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[Anomalous origin of the left coronary artery. Presentation of 5 cases]
Insights
Anomalous left coronary artery from the pulmonary trunk causes significant ventricular dysfunction in infants. Early diagnosis via echocardiography and angio-haemodynamic assessment is crucial for appropriate treatment.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiovascular Imaging
Context:
- Anomalous left coronary artery originating from the pulmonary trunk (ALCAPA) is a rare congenital heart defect.
- It typically presents in infancy with symptoms of heart failure due to myocardial ischemia.
- Accurate diagnosis and assessment of ventricular function are critical for management.
Purpose:
- To report clinical findings in five cases of ALCAPA.
- To evaluate ventricular function using echocardiography and angio-haemodynamic methods.
- To correlate diagnostic findings with treatment decisions.
Summary:
- Five infants with ALCAPA underwent echocardiographic and angio-haemodynamic evaluation at 7-8 months of age.
- Echocardiography revealed left atrial and ventricular dilatation, with diminished ventricular function.
- Quantitative angiography showed increased end-diastolic volumes and reduced ejection phase indexes.
- Myocardial perfusion defects were noted in one case via thallium-201 imaging.
- Electrocardiography, echocardiography, and angio-haemodynamic studies supported the diagnosis and assessment of myocardial pump function.
Impact:
- Highlights the diagnostic utility of echocardiography and angio-haemodynamic studies in ALCAPA.
- Emphasizes the importance of assessing myocardial pump function for guiding treatment.
- Provides insights into the clinical presentation and functional consequences of this rare anomaly.
Abstract:
Clinical findings are reported in 5 cases of anomalous left coronary artery originating from pulmonary trunk, with reference of ventricular function evaluated through echocardiographic and angio-haemodynamic methods. All patients were catheterized between 7 and 8 months of age. Left atrial and ventricular dilatation, diminished shortening of minor diameter and free wall thickness and septum normal values were obtained by echocardiography. Quantitative angiography of left ventricle demonstrated an elevation of end-diastolic volumes (mean values: 270% of predicted normal). The ejection phase indexes are diminished. The left ventricular mass/end-diastolic volume ratio was low in all cases. Myocardial imaging with thallium-201 demonstrated perfusion defect in lateral area of left ventricle in one patient. The electrocardiographic signs of myocardial necrosis support the diagnosis, and the echocardiographic and isotopic studies may contribute to it. The angio-haemodynamic method corroborate the diagnosis, and the myocardial pump function evaluation may help to decide the most appropriate treatment for this anomaly.